AB0622 The Joint Vasculitis Registry in German-speaking countries (GeVas) – subgroup analysis of 113 GPA-patients
Authors: S. Arnold, P. Wallmeier, F. Schubach, G. Ihorst, P. Aries, R. Bergner, J. P. Bremer, N. Görl, B. Hellmich, J. Henes, B. Hoyer, A. Kangowski, I. Kötter, C. Metzler, U. Müller-Ladner, M. Schaier, U. Schönermark, J. Thiel, L. Unger, N. Venhoff, J. Weinmann-Menke, J. Petersen, C. Iking-Konert, P. Lamprecht
Journal: Annals of the Rheumatic Diseases (2022)

Background Granulomatosis with polyangiitis (GPA) is the second most frequent vasculitis in Germany with an annual incidence of 34 per million and a prevalence of 210 per million [1]. GPA is characterized by its chronic course, frequent relapses, significant overall morbidity and mortality, and substantial socio-economic impact. Multiorgan involvement affecting the respiratory tract, kidney, and other organs is common. Limited variants also occur [2]. So far, prospective long-term observational data on the disease course of GPA are missing in Germany. Therefore, the Joint Vasculitis Registry in German-speaking countries (GeVas) has been established to follow the course of patients recently diagnosed with vasculitis or a change of their treatment due to a relapse (inception cohort). The GeVas registry allows long-term follow-up of a substantial cohort of vasculitis patients in a multicenter setting. Objectives To present the first data on the follow-up of newly diagnosed and relapsing GPA enrolled in the GeVas registry. Methods GeVas is a prospective, web-based, multicenter, clinician-driven registry for the documentation of organ manifestations, damage, long-term outcomes, and therapy regimens in various types of vasculitis. Recruitment started in June 2019. By January 2022, 17 centers in Germany were initiated and started enrolling patients. Meanwhile, more than 350 patients have been documented in the registry. Sites in Austria and the German-speaking cantons of Switzerland will be integrated soon [3]. Results By mid-October 2021, the participating centers included 113 patients with GPA. The majority of patients were PR3-ANCA positive and affected by general symptoms, ENT, lung, renal, and neurological involvement. Patients commonly received cyclophosphamide or rituximab in combination with glucocorticoids for the induction of remission. Fewer patients received methotrexate or other immunosuppressants. Patient characteristics and therapy are summarized in Table 1. Table 1. Patient characteristics (n = 113). *Unless otherwise specified. Category Feature n (%)* Age Age (years); median [range] 60 [51 - 70] Gender Male 61 (54.0) Female 52 (46.0) Reason for inclusion in the registry Newly diagnosed vasculitis 57 (51.4) Relapse 56 (49.6) ANCA status PR3-ANCA 99 (87.6) MPO-ANCA 4 (3.6) ANCA negative 9 (7.9) Organ manifestation General symptoms 86 (76.1) ENT 69 (61.1) Lung/chest 66 (58.4) Renal 35 (31.0) Cardiovascular 7 (6.2) GI 3 (2.7) Neurological 27 (23.9) Therapy Glucocorticoid 102 (90.3) Rituximab 56 (49.6) Cyclophosphamide 37 (32.7) Methotrexate and other immunosuppressants, respectively 26 (23.0) and 19 (16.8), respectively Conclusion Here, we present the first interim analysis of the GeVas registry. Clinical manifestations of GPA reported herein show less frequent renal involvement in comparison with a recent report from another European registry (POLVAS) and an UK study [4, 5]. This is potentially related to the predominance of recruiting rheumatology centers thus far. By contrast, respiratory tract involvement is more frequent and PR3-ANCA less common in Japan [5]. Further data are prospectively documented and a follow up analysis is in progress. References [1]Hellmich B, et al. New insights into the epidemiology of ANCA-associated vasculitides in Germany: results from a claims data study. Rheumatology 2021;60:4868-73. [2]Kitching AR, et al. ANCA-associated vasculitis. Nat Rev Dis Primers 2020;6:71. [3]Iking-Konert C, et al. The Joint Vasculitis Registry in German-speaking countries (GeVas) – a prospective, multicenter registry for the follow-up of long-term outcomes in vasculitis. BMC Rheumatol 2021;5:40. [4]Wójcik K, et al. Clinical characteristics of Polish patients with ANCA-asscoiated vasculitides – retrospective analysis of POLVAS registry. Clin Rheumatol 2019;38:2553-63. [5]Furuta S, et al. Comparison of the phenotype and outcome of granulomatosis with polyangiitis between UK and Japanese cohorts. J Rheumatol 2017;44:216-22. Acknowledgements GeVas was supported by unrestricted grants by: DGRh, John Grube Foundation, Vifor and Roche Pharma Disclosure of Interests Sabrina Arnold: None declared, Pia Wallmeier: None declared, Fabian Schubach: None declared, Gabriele Ihorst: None declared, Peer Aries: None declared, Raoul Bergner Consultant of: VIFOR, Jan Philip Bremer: None declared, Norman Görl: None declared, Bernhard Hellmich: None declared, Jörg Henes: None declared, Bimba Hoyer: None declared, Antje Kangowski: None declared, Ina Kötter: None declared, Claudia Metzler: None declared, Ulf Müller-Ladner: None declared, Matthias Schaier: None declared, Ulf Schönermark: None declared, Jens Thiel: None declared, Leonore Unger: None declared, Nils Venhoff Speakers bureau: Roche and Vifor: speaker honoraries, Consultant of: Roche and Vifor: advisory boards, Grant/research support from: John-Grube Research Award 2021, Julia Weinmann-Menke: None declared, Jana Petersen: None declared, Christof Iking-Konert Speakers bureau: Lecture fees from: Chugai, GSK, Roche, and Vifor, Consultant of: Consulting fees from: Chugai, GSK, Roche, and Vifor, Grant/research support from: Research grants for GeVas: Roche, Vifor, DGRh, John Grube Foundation, Peter Lamprecht Speakers bureau: Chugai, GSK, Roche, and Vifor, Consultant of: Chugai, GSK, Roche, and Vifor, Grant/research support from: DGRh, John Grube Foundation, Roche, and Vifor